Abstract
Glomerular diseases are a common cause of chronic kidney and end-stage renal disease in the pediatric population. Many of these patients will need dialysis and kidney transplantation when they reach adolescence or adulthood. This chapter begins with a diagnostic approach to hematuria and proteinuria. Primary nephrotic syndrome is the most common glomerular disorder in children. Common aspects of nephrotic syndrome are described followed by minimal change disease, focal segmental glomerulosclerosis and inherited forms of nephrotic syndrome. A nephritic syndrome is characterized by hematuria and proteinuria, edema, hypertension and diminished glomerular filtration rate with or without oliguria. Descriptions of acute postinfectious, immune complex glomerulonephritides, and rapidly progressive glomerulonephritides (GN) are followed by examples of chronic GN including membranoproliferative GN, IgA nephropathy, membranous and C1q nephropathy. Vasculitides affecting the kidney include Schönlein–Henoch nephritis, ANCA-associated vasculitis and lupus nephritis. The chapter concludes with thrombotic microangiopathies, emphasizing typical and atypical forms of hemolytic uremic syndrome (HUS) and inherited, structural disorders of the glomerular basement membrane, Alport syndrome and thin basement membrane nephropathy.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Suggested Reading
Ariceta G et al (2009) European Paediatric Study Group for HUS. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol 24:687–696
Benbassat J, Gergawi M, Offringa M, Drukker A (1996) Symptomless microhaematuria in schoolchildren: causes for variable management strategies. QJM 89:845–854
Bergstein J, Leiser J, Andreoli S (2005) The clinical significance of asymptomatic gross and microscopic hematuria in children. Arch Pediatr Adolesc Med 159:353–355
Bolton WK (2000) Treatment of RPGN. Semin Nephrol 3:244–255
Cattran D (2005) Management of membranous nephropathy. When and what for treatment. JASN 16(5):1188–1194
Coppo R, Troyanov S, Camilla R et al (2010) The Oxford IgA nephropathy clinic-pathological classification is valid for children as well as adults. Kidney Int 77:921–927
Dillon MJ, Elefhtheriou D, Brogan PA (2010) Medium vessel vasculitis. PediatrNephrol 25:1641–1652
EdströmHalling S, Söderberg MP, Berg UB (2012) Predictors of outcome in paediatric IgA nephropathy with regard to clinical and histopathological variables (Oxford classification). Nephrol Dial Transplant 27:715–722
Gbadegesin R, Lavin P, Foreman J, Winn M (2011) Pathogenesis and therapy of focal segmental glomerulosclerosis: an update. Pediatr Nephrol 26(7):1001–1015
Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, Wigfall D, Miles P, Powell L, Lin JJ, Trachtman H, Greenbaum LA (2009) Management of childhood onset nephrotic syndrome. Pediatrics 124:747–757
Glassock RJ (2003) Diagnosis of membranous nephropathy. Semin Nephrol 23(4):324–332
Hahn BH et al (2012) American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritis. Arthritis Care Res 64:797–808
Jauhola O, Ronkainen J, Autio-Harmainen H, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, Jahnukainen T, Rajantie J, Ormälä T, Nuutinen M (2011) Cyclosporine A vs. methylprednisolone for Henoch-Schönlein nephritis: a randomized trial. Pediatr Nephrol 26:2159–2166
Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T, Jahnukainen T, Rajantie J, Örmälä T, Nuutinen M (2012) Outcome of Henoch-Schönlein purpura 8 years after treatment with a placebo or prednisone at disease onset. Pediatr Nephrol 27:933–939
Jennette JC et al (2012) Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheumat 2013; 65:1–11
Kanjanabuch T, Kittikowit W, Eiam-Ong S (2009) An update on acute postinfectious glomerulonephritis worldwide. Nat Rev Nephrol 5:259–269
Kashtan C, Ding J, Gregory M, Heidet L, Knebelman B, Rheault M, Licht C (2013) Clinical practice recommendations for the treatment of Alport syndrome: a statement of the Alport Syndrome Research Collaborative. Pediatr Nephrol 28(1):5–11
Kidney Disease: Improving Global Outcomes (2012) KDIGO practice guideline for glomerulonephritis. Kidney Int Suppl 2:
Lombel RM, Gipson DS, Hodson EM (2013a) Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol 28(3):415–426
Lombel RM, Hodson EM, Gipson DS (2013b) Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO. Pediatr Nephrol 28(3):409–414
Nakopoulou L (2001) Membranoproliferative glomerulonephritis. Nephrol Dial Transplan 16(6):71–73
Ozen S et al (2010) Paediatric Rheumatology International Trials Organisation (PRINTO). EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 69:798–806
Pallan L, Savage CO, Harper L (2009) ANCA associated vasculitis: From bench research to novel treatments. Nat Rev Nephrol 5:278–286
Plank C, Kalb V, Hinkes B, Hildebrandt F, Gefeller O, Rascher W (2008) For Arbeitsgemeinschaft für Pädiatrische Nephrologie. Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome—a randomized controlled multicentre trial by the Arbeitsgemeinschaft für Pädiatrische Nephrologie. Pediatr Nephrol 23:1483–1493
Quigley R (2008) Evaluation of hematuria and proteinuria: how should a pediatrician proceed? Curr Opin Pediatr 20:140–144
Ren P, Han F, Chen L, Xu Y, Wang Y, Chen J (2012) The Combination of Mycophenolate Mofetil with Corticosteroids Induces Remission of Henoch-Schönlein Purpura Nephritis. Am J Nephrol 36:271–277
Tsai HM (2013) Thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome - an update. Hematol Oncol Clin N Am 27:565–584
Tumlin JA, Lohavichan V, Hennigar R (2003) Crescentic, proliferative IgA nephropathy: clinical and histological response to methylprednisolone and intravenous cyclophosphamide. Nephrol Dial Transplant 18:1321–1329
Tune BM, Lieberman E, Mendoza SA (1996) Steroid-resistant nephrotic focal segmental glomerulosclerosis: a treatable disease. Pediatr Nephrol 10:772–778
van Husen M, Kemper MJ (2011) New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol 26(6):881–892
Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M, International Society of Nephrology Working Group on the Classification of Lupus Nephritis, Renal Pathology Society Working Group on the Classification of Lupus Nephritis (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 65:521–530
Wenderfer SE, Swinford RD, Braun MC (2010) C1q nephropathy in the pediatric population: pathology and pathogenesis. Pediatr Nephrol 25:1385–1396
Wong CS, Fink CA, Baechle J, Harris AA, Staples AO, Brandt JR (2009) C1q nephropathy and minimal change nephrotic syndrome. Pediatr Nephrol 24:761–767
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Bitzan, M. (2014). Glomerular Diseases. In: Phadke, K., Goodyer, P., Bitzan, M. (eds) Manual of Pediatric Nephrology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-12483-9_3
Download citation
DOI: https://doi.org/10.1007/978-3-642-12483-9_3
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-12482-2
Online ISBN: 978-3-642-12483-9
eBook Packages: MedicineMedicine (R0)